A Devastating Disease Makes These Children's Skin as Fragile as a Butterfly's Wings

A rare health condition that specialists call "the worst disease you've never heard of" was recently highlighted in a Washington Post article that profiles two children who were born with it, including 13-month-old Elizabeth* Federici and 10-year-old Ella Murray. The disease, epidermolysis bullosa (EB), is a rare genetic connective tissue disorder in which cells lack a protein needed for skin layers to adhere, causing skin vulnerable to tears and blisters in response to the most minor friction, and leaving patients in constant pain and discomfort from itchy scarring, according to Dystrophic Epidermolysis Bullosa Research Association of America (DEBRA).

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Every morning and every evening, Kevin and Heather Federici drain the blisters and clean the wounds on their daughter Elizabeth’s body.

Katherine Frey/The Washington Post via Getty Images

Experts call infants born with EB "butterfly babies" since their skin is as fragile as a butterfly's wings. Caring for butterfly children involves the painful popping of their blisters to prevent them from spreading and damaging more skin, as well as bandaging the open wounds that result from blister-popping to prevent infection. The process can take up to five hours per session twice a day, making it not just time-consuming, but emotionally wrenching for parents who must inflict pain on their kids to protect them. Treating EB is also expensive, since medical supplies can cost up to $1,000 per month.

Exhausted, hot, and hurting, Ella Murray sighs as she hangs over the side of the bathtub.

Katherine Frey/The Washington Post via Getty Images

EB has no known cure, and irritants as benign as a sock seam, bowel movement, or change in temperature can cause severe blistering and scarring so bad it can ultimately fuse fingers and toes together. Protecting children born with EB and treating inevitable wounds is really the only option.

Just 1 in 20,000 people — or 200 American babies a year — are born with EB, according to DEBRA, making the condition so rare that it continues to confound many medical professionals. It can be inherited or result from genetic mutation. To complicate things even more, severity can range from so mild you don't know you've got EB, to a life marked by full-body scarring and a high likelihood of potentially terminal skin cancer by the time a patient reaches his or her early 30s.

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While EB caregivers tend to live in fear of their children becoming infected, and worry their scarred children will be shunned by a world that hasn't heard of and can't possibly understand what it's like to cope with this disease, the outlook for patients with EB isn't always bleak: Elizabeth's parents are optimistic that things will get easier for their daughter as she ages, particularly after tests revealed that this particular kind of EB only affects the upper skin layer and shouldn't stop their daughter from reaching a normal life expectancy, according to the Washington Post.

Meanwhile, the Washington Post also reports that Ella, who has a more severe case of EB, is navigating fourth grade with impressive grades and hobbies like singing, reading, and journalism despite her reliance on a feeding tube, which delivers extra the calories her body needs to heal its wounds.

Ella Murray during her fourth grade class at her elementary school.

Katherine Frey/The Washington Post via Getty Images

Ella's fingers are fused by scar tissue, her toes are webbed, she needs esophageal surgery every year to prevent internal blisters from hindering her ability to swallow, and will eventually need surgery to maintain use of her thumbs. Still, her parents are managing and optimistically expecting their third child, who's unlikely to be born with EB according to genetic testing.

Until experts unearth an EB cure, DEBRA welcomes donations for ongoing research at Debra.Org/Donate.

*Elizabeth is the Federicis' daughter's middle name. She's identified as such to protect her privacy.

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